Fig. 7

Several imaging findings in a male patient with deficiency of interleukin 1 receptor antagonist (DIRA). a Posteroanterior view of the mandible at the age of 7 years when he presented with left mandibular swelling shows a well-defined lytic lesion within the body of the left mandible with expansion of the bone and thinning of the cortex (arrow). There are no aggressive features. b Axial unenhanced CT of the chest in bone window demonstrates diffuse broadening of the ribs with ground glass density of the medulla and thinning of the cortex. Bone biopsy showed fibrous changes indicative of healing that is likely related to the patient’s bone disease. There are alveolar (black arrow) and interstitial (long white arrow) pulmonary opacities and nodules with cyst formation (short white arrow). Lung biopsy: pulmonary hemosiderosis. c Anteroposterior radiograph of the hands performed when the patient presented with abnormal nails demonstrates multiple bilateral symmetrical metaphyseal lytic lesions with associated cortical thinning (arrows). There is distal phalangeal soft tissue thickening